Primary Sclerosing Cholangitis
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The Correct Answer is A. Liver biopsy is diagnostic in most casesThe best way to diagnose PSC is by ERCP, not by a liver biopsy because liver biopsy is sort of a random event.
Primary Sclerosing Cholangitis:
It is a set of disorders where there is fibrosis of the bile duct. It causes diffuse stricturing of the biliary tree and is insidious. It comes on slowly, people do not even know they have it. The key thing is that it occurs in inflammatory bowel disease and there are two major inflammatory bowel diseases. There is Crohn's disease, ileitis and then ulcerative colitis. The ulcerative colitis is the one that is really associated with this situation. There are autoimmune and collagen-vascular diseases that are associated with this as well. It can be seen in autoimmune types of things such as hepatic allograft rejections that can cause sclerosing cholangitis.
There can be infiltrated diseases such as histiocytosis sarcoid eosonophilic cholangeitis and immunodeficiency diseases. The age range is somewhere around 25-45. Females predominate over males, but it is equal if inflammatory bowel disease is not involved.
Presentation:
It usually presents with jaundice because it is insidious. So people feel fine, and all of a sudden somebody tells them they look jaundiced and they come in and see the physician. Pruritus occurs in 1-2/3 of the cases related to obstruction of bile salt excretion. The patients who have that obstruction, there is a significant number of them, they develop severe pruritus. But once the obstruction is relieved it goes away. We see people with stone disease, obstructions and tumors in the bile duct and obstruction may come in with severe pruritus and claw marks all over their bodies. After we relieve the obstruction that disappears.
The major problem with sclerosing cholangitis even though it leads cirrhosis, and liver transplantation is that it has a high association with cholangiocarcinoma. That is a primary cancer of the bile duct. It has very high association 30-40%. There may be an associated increase in colon cancers associated with the dual entity of PSC and ulcerative colitis. With a liver transplant there is an excellent five-year survival rate of 85-92%, but that is in case if these patients do not have cholangiocarcinoma.
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